肝原发性血管肉瘤临床病理分析

 目的 探讨肝原发性血管肉瘤（PHA）的临床病理学与免疫组织化学特点。方法 采用HE和免疫组织化学SP法染色对3例PHA进行研究，观察PHA的临床病理学、免疫组织化学特点。结果 患者年龄2～68岁，男∶女为2∶1；临床症状和实验室检查无特异性。1例为多个结节，2例为单个结节；肿瘤直径平均大小为7.2（5～11.3）cm，有出血和坏死。病理学特征：肿瘤细胞呈梭形和不规则形，大小不一。胞质较少，嗜酸性，淡染,边界不清，核较大，卵圆形或梭形，染色深，核仁大小不一，可见少数核分裂象，瘤细胞部分排列成索状，片状，较密集，与周围肝组织界线不清，部分区域可见大而不规则的血管腔隙，局部肿瘤细胞集聚成团，呈乳头状突入血管腔内，相互吻合，内衬不典型性细胞。不规则血管腔样结构广泛弥漫分布于肝实质内，伴局部出血。免疫组化因子Ⅷ和（或）CD34阳性。结论 PHA极少见，为高度恶性，预后差。临床和病理上均易误诊为肝血管瘤、肝上皮样血管内皮瘤、肝细胞癌等；根据PHA的组织学和免疫组化特点，可作出明确的病理诊断。

Clinicopathological study on primary hepatic angiosarcoma

Objective To study the clinicopathological and immunohistochemical features of Primary Hepatic Angiosarcoma（PHA）. Method H-E and immunohistochemistry were used to study 3 cases of PHA. Results The patients were 2～68 years old, with male to female ratio being 2/1, and presented with nonspe－cific symptoms. The lesion was multinodular in 1 case and uninodular in 2 cases. The median size was 7.2 cm (5～11.3 cm), accompanied by hemorrhage and necrosis. Histological, there was a wide morphologic spectrum among cases. 3 cases of PHA included areas similar to cavernous and capillary hemangioma，spindle cell and epithelioid sarcoma. The spindle, rounded or irregular tumor cells of relatively high nuclear grade were ar－ranged in sheets, small nests, cords or rudimentary vascular channels, and displayed mitotic activity. The vas－cular channels were irregular in shape, freely intercommunicate with one another in a sinusoidal fashion, and infiltrated adjacent hepatic tissues in a destructive fashion. In some areas the vascular channels were lined by a surfeit of neoplastic endothelium forming intraluminal buds, projections or papillae. Immunohistochemically, tumor cells were positive for factor Ⅷ-related antigen and CD34. Conclusions PHA is an extremely rare, highly malignant hepatic tumor with poor outcome. Surgical resection can obtain prolonged survival. PHA may be clinically or histological misdiagnosed as hepatic hemangioma, hepatic epithelioid hemangioendothelioma or hepatic cell carcinoma. The diagnosis can be made based on histological and immunohistochemical features of the tumor.